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- Title
Sporadic Creutzfeldt-Jakob disease in adults over 80 years: a 10-year review of United Kingdom surveillance.
- Authors
McDermott, Eugene Ace; Watson, Neil; Tam, Johnny; Centola, John; King, Hatice Kurucu; Mackenzie, Jan; Summers, David; Green, Alison; Barria, Marcelo A; Smith, Colin; Pal, Suvankar
- Abstract
Introduction Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease with public health implications. Mean age of onset is 68 years. Age-specific incidence declines after 80 years. This may arise from under-ascertainment or other biological features of the disease. Accurate characterisation of late-onset sCJD is important for early diagnosis, avoiding unnecessary investigations and improving ascertainment for public health purposes. Objective To phenotype the clinical features and investigation profile of sCJD in adults >80 years. Methods We analysed all probable and definite sCJD cases identified by the UK National CJD Research & Surveillance Unit over a 10-year period (2011–2021). Individuals were grouped by age of onset. Clinical features and investigation profiles were compared. Results 10.3% (123/1196) had an age of onset over 80. Median survival was shorter (3.2 vs 4.3 months; P < 0.001). Pyramidal signs (48.3% vs 34.2%; P = 0.008) and akinetic mutism (55.1% vs 33.2%; P < 0.001) were more frequent. Psychiatric symptoms (26.3% vs 39.6%; P = 0.01) and cerebellar signs (65.4% vs 78.6%, P = 0.007) were less frequent. Cognitive impairment and myoclonus were highly prevalent regardless of age. Between age groups, the diagnostic sensitivity of cerebrospinal fluid real-time quaking-induced conversion (CSF RT-QuIC) (92.9% vs 91.9%, P = 0.74) was comparable, electroencephalography was superior (41.5% vs 25.4%; P = 0.006) and MRI was inferior (67.8% vs 91.4%; P < 0.001). Conclusions Late-onset sCJD has distinct clinical features, shorter survival and a different profile of investigation sensitivity. CSF RT-QuIC, MRI brain and specialist CJD review is recommended in older adults with a rapidly progressive neurological disorder. Autopsy is valuable when the cause remains elusive.
- Subjects
UNITED Kingdom; CREUTZFELDT-Jakob disease diagnosis; PUBLIC health surveillance; MYOCLONUS; CREUTZFELDT-Jakob disease; AUTOPSY; DISEASE duration; RESEARCH funding; ELECTROENCEPHALOGRAPHY; MAGNETIC resonance imaging; DESCRIPTIVE statistics; AGE factors in disease; LONGITUDINAL method; COGNITION disorders; SURVIVAL analysis (Biometry); PHENOTYPES; MUTISM; CEREBROSPINAL fluid; SENSITIVITY &; specificity (Statistics); SYMPTOMS; OLD age
- Publication
Age & Ageing, 2024, Vol 53, Issue 5, p1
- ISSN
0002-0729
- Publication type
Article
- DOI
10.1093/ageing/afae086